Congenital adrenal hyperplasia Health Dictionary

Also known as suprarenal glands, these are two small triangular ENDOCRINE GLANDS situated one upon the upper end of each kidney. (See diagram of ABDOMEN.)

Structure Each suprarenal gland has an enveloping layer of ?brous tissue. Within this, the gland shows two distinct parts: an outer, ?rm, deep-yellow cortical (see CORTEX) layer, and a central, soft, dark-brown medullary (see MEDULLA) portion. The cortical part consists of columns of cells running from the surface inwards, whilst in the medullary portion the cells are arranged irregularly and separated from one another by large capillary blood vessels.

Functions Removal of the suprarenal glands in animals is speedily followed by great muscular prostration and death within a few days. In human beings, disease of the suprarenal glands usually causes ADDISON’S DISEASE, in which the chief symptoms are increasing weakness and bronzing of the skin. The medulla of the glands produces a substance – ADRENALINE – the effects of which closely resemble those brought about by activity of the SYMPATHETIC NERVOUS SYSTEM: dilated pupils, hair standing on end, quickening and strengthening of the heartbeat, immobilisation of the gut, increased output of sugar from the liver into the bloodstream. Several hormones (called CORTICOSTEROIDS) are produced in the cortex of the gland and play a vital role in the metabolism of the body. Some (such as aldosterone) control the electrolyte balance of the body and help to maintain the blood pressure and blood volume. Others are concerned in carbohydrate metabolism, whilst others again are concerned with sex physiology. HYDROCORTISONE is the most important hormone of the adrenal cortex, controlling as it does the body’s use of carbohydrates, fats and proteins. It also helps to suppress in?ammatory reactions and has an in?uence on the immune system.... adrenal glands

Congenital deformities, diseases, etc. are those which are either present at birth, or which, being transmitted direct from the parents, show themselves some time after birth.... congenital

Hyperplasia means an abnormal increase in the number of cells in a tissue.... hyperplasia

Heart disease arising from abnormal development. Some cases are hereditary, others due to drugs taken during pregnancy. Many owe their origin to illnesses of the mother such as German measles. Structural abnormalities of the heart take different forms but whatever the case, when under abnormal pressure and stress, all may derive some small benefit from the sustaining properties of Hawthorn berry and other phytomedicines.

Alternatives. To sustain.

Teas. Lime flowers, Motherwort, Buckwheat, Hawthorn.

Tablets/capsules. Hawthorn, Mistletoe, Motherwort.

Formula. Hawthorn 2; Lily of the Valley 1; Selenicereus grandiflorus 1. Powders: 500mg (two 00 capsules or one-third teaspoon). Liquid extracts: 1 teaspoon. Tinctures: 2 teaspoons. In water morning and evening. ... heart disease – congenital

(BPH) A medical term for enlargement of the prostate gland (see prostate, enlarged).... benign prostatic hyperplasia

The outer covering of the two adrenal glands that lie atop each kidney. Embryonically derived from gonad tissue, they make steroid hormones that control electrolytes, the management of fuels, the rate of anabolism, the general response to stress, and maintenance of nonspecific resistance.... adrenal cortex

The inner part of the adrenals, derived embryonically from spinal nerve precursors, they secrete epinephrine, norepinephrine and dopamine; used locally as neurotransmitters, sensitive receptors can be mobilized totally by the adrenal medullas.... adrenal medulla

(BPH) The benign buildup in the prostate of “warts” or epithelial neoplasias that can block or interrupt urination, and which are usually concurrent with moderate prostate enlargement. They cause a dull ache on urination, ejaculation, and/or defecation. The diagnosis is medical, since the same subjective conditions can result from cancer of the prostate. BPH is common in men over fifty and can be the result either of diminished production of complete testosterone or poor pelvic circulation. Alcohol, coffee, speed, and antihistamines can all aggravate the problem.... benign prostatic hypertrophy, or hyperplasia

An agent which stimulates the adrenal glands thereby increasing secretion of cortisol and adrenal hormones. A herb with a mild cortico-steroid effect. Liquorice. Ginseng. Sarsaparilla. ... adrenal-activator

Insufficient production of hormones by the adrenal cortex (the outer part of the adrenal glands).

It can be acute or chronic.

Adrenal failure may be caused by a disorder of the adrenal glands, in which case it is called Addison’s disease, or by reduced stimulation of the adrenal cortex by ACTH, a hormone produced by the pituitary gland.... adrenal failure

Cancerous or noncancerous tumours in the adrenal glands, usually causing excess secretion of hormones. Adrenal tumours are rare. Tumours of the adrenal cortex may secrete aldosterone, causing primary aldosteronism, or hydrocortisone, causing Cushing’s syndrome. Tumours of the medulla may cause excess secretion of adrenaline and noradrenaline. Two types of tumour affect the medulla: phaeochromocytoma and neuroblastoma, which affects children. These tumours cause intermittent hypertension and sweating attacks. Surgical removal of a tumour usually cures these conditions.... adrenal tumours

The separation of a body part (usually a limb, finger, or toe) from the rest of the body, as a result of the part’s blood supply being blocked by a band of amnion (fetal membrane) in the uterus.

The affected part may be completely separated or show the marks of the “amniotic band” after birth.

(See also limb defects.)... amputation, congenital

See hyperplasia, gingival.... gingival hyperplasia

A range of uncommon but sometimes serious disorders due to deficient or excessive production of hormones by one or both of the adrenal glands.

A genetic defect causes congenital adrenal hyperplasia, in which the adrenal cortex is unable to make sufficient hydrocortisone and aldosterone, and androgens are produced in excess. In adrenal failure, there is also deficient production of hormones by the adrenal cortex; if due to disease of the adrenal glands, it is called Addison’s disease. Adrenal tumours are rare and generally lead to excess hormone production.

In many cases, disturbed activity of the adrenal glands is caused, not by disease of the glands themselves, but by an increase or decrease in the blood level of hormones that influence the action of the adrenal glands. For example, hydrocortisone production by the adrenal cortex is controlled by ACTH, which is secreted by the pituitary gland. Pituitary disorders can disrupt production of hydrocortisone.... adrenal gland disorders

An uncommon genetic disorder in which an enzyme defect blocks the production of corticosteroid hormones from the adrenal glands. Excessive amounts of androgens (male sex hormones) are produced, which can result in abnormal genital development in an affected fetus. Other effects include dehydration, weight loss, low blood pressure, and hypoglycaemia. Hyperplasia (enlargement) of the adrenal glands occurs and there is excessive skin pigmentation in skin creases and around the nipples.

In severe cases, the disorder is apparent soon after birth. In milder cases, symptoms appear later, sometimes producing premature puberty in boys and delayed menstruation, hirsutism, and potential infertility in girls.

Congenital adrenal hyperplasia is confirmed by measuring corticosteroid hormones in blood and urine. Treatment is by hormone replacement. If this is started early, normal sexual development and fertility usually follow.... adrenal hyperplasia, congenital

See developmental hip dysplasia.... hip, congenital dislocation of

Abnormal enlargement of the gums.

Causes include gingivitis, persistent breathing through the mouth, the anticonvulsant drug phenytoin, and ill-fitting dentures.

Surgical treatment may be needed.... hyperplasia, gingival

Infection acquired in the uterus or during birth. Many microorganisms can pass from the mother, by way of the placenta, into the circulation of the growing fetus. Particularly serious infections acquired in the uterus are rubella, syphilis, toxoplasmosis, and cytomegalovirus; all these infections may cause intrauterine growth retardation. Rubella that occurs in early pregnancy may cause deafness, congenital heart disease, and eye disorders. Some infections in later pregnancy, particularly with a herpes virus, may also damage the fetus severely. A woman infected with HIV risks passing on the virus to her baby during pregnancy, but the risk can be reduced by use of antiretroviral drugs during the pregnancy.

Infections acquired during birth are almost always the result of microorganisms in the mother’s vaginal secretions or uterine fluid. Premature rupture of the membranes is associated with increased risk of infection, particularly streptococcal. Conditions that can be acquired during delivery include herpes, chlamydial infections, and gonorrhoea.

Treatment of the baby depends on the type of infection. Some birth defects caused by infection (such as certain types of heart defect) can be treated; others (such as congenital deafness) are usually not treatable.... infection, congenital

(CDH) herniation of the fetal abdominal organs into the fetal chest, which occurs in one in 2000–5000 live births. This leads to pulmonary *hypoplasia, which is the main cause of the associated high neonatal mortality. The risk of pulmonary hypoplasia is substantially greater where there is herniation of the liver into the thoracic cavity. CDH is commonly associated with additional structural abnormalities (cardiac, neural tube defects, and exomphalos), and the risk of chromosomal abnormality (*aneuploidy) is 10–20%. Demonstration of a fluid-filled bowel at the level of the heart on ultrasound is diagnostic.... congenital diaphragmatic hernia

a hereditary condition (inherited as an autosomal *recessive) causing severe visual loss in infants. The *fundus usually appears to be normal when examined with an *ophthalmoscope, but marked abnormalities are found on the ERG (see electroretinography), usually with extinguished wave pattern. [T. Leber (1840–1917), German ophthalmologist]... leber’s congenital amaurosis

a pathological process involved in *atherosclerosis of arteries and vein grafts, and in *restenosis, that may be in response to angioplasty and stent placement. Damage to the endothelium of the artery exposes the underlying smooth muscle cells in the *media to cytokines, growth factors, and other plasma components in the circulation, which results in loss of their contractile characteristics. These abnormal muscle cells migrate to the *intima, where they proliferate and eventually form a thick layer of tissue (neointima), which occludes the artery.... neointimal hyperplasia

(CDH) an abnormality present at birth in which the head of the femur is displaced or easily displaceable from the acetabulum (socket) of the ilium, which is poorly developed; it frequently affects both hip joints. CDH occurs in about 1.5 per 1000 live births, being more common in first-born girls, in breech deliveries, and if there is a family history of the condition. The leg is shortened and has a reduced range of movement, and the skin creases may be asymmetrical. All babies are routinely screened for CDH at birth and at developmental check-ups by gentle manipulation of the hip causing it to be reduced and dislocated with a clunk (see Barlow manoeuvre; Ortolani manoeuvre). The diagnosis is confirmed by X-ray or ultrasound scan. Treatment is with a special harness holding the hip in the correct position. If this is unsuccessful, the hip is reduced under anaesthetic and held with a plaster of Paris cast or the defect is corrected by surgery. Successful treatment of an infant can give a normal hip; if the dislocation is not detected, the hip does not develop normally and osteoarthritis develops at a young age.... congenital dislocation of the hip

an increase in the thickness of the cells of the *endometrium, usually due to prolonged exposure to unopposed oestrogen, which can be endogenous, as in anovular menstrual cycles; or exogenous, deriving, for example, from *hormone replacement therapy or an oestrogen-secreting tumour. It is classified as simple, complex, or atypical. Endometrial hyperplasia most commonly presents with abnormal uterine bleeding and accounts for 15% cases of postmenopausal bleeding. It may also be asymptomatic, and in some cases regresses spontaneously without ever being detected. The presence of atypical cells may lead to *endometrial cancer. Treatment can include progestogen therapy or surgery (see endometrial ablation); hysterectomy is advised when atypical changes are present.... endometrial hyperplasia